PB2319: ACQUIRED COAGULOPATHY: FACTOR X DEFICIENCY AT DIAGNOSIS OF MULTIPLE MYELOMA WITHOUT ASSOCIATED AMYLOIDOSIS
Authors: M. De La Torre De La Paz, G. Figaredo García Mina, A. Guerrero Díez, A. Rodríguez Alén, M. D. L. O. Abío Calvete, N. Rollón Simón, J. Cuesta Tovar
Journal: HemaSphere (2022)

Background: Plasma cell dyscrasias, including Multiple Myeloma (MM), are frequently associated with alterations in hemostasis tests and to a lesser extent with the presence of coagulopathy. Acquired factor X deficiency is usually associated with the presence of amyloidosis (AL) and very rarely appears in patients with MM without AL. Aims: The aim of this work is to report the presence of acquired coagulopathy at the debut of Multiple Myeloma, specifically the presence of an acquired factor X deficiency. Methods: Description of a clinical case diagnosed in our haematology service about the acquired FX deficiency at diagnosis of a patient with MM without AL Results: A 77-year-old patient was diagnosed with MM IgG Lambda. Initial laboratory studies revealed an alteration in the hemostasis tests, not previously present, evidencing an acquired origin. She presented with prolonged PT and aPTT measurements, which corrected after incubation with normal plasma. Thrombin Time (TT) did not show alterations. FX was 19%. Given the frequent association between this deficit and AL, abdominal fat pad biopsy was performed, which was negative for Congo red staining. Treatment was started correcting the coagulopathy during this period. In 2021, she suffered an episode of epistaxis and she needed hospital admission. A relapse of his underlying disease and secondary worsening of the coagulopathy were observed, with a FX level of 8%. Furthermore, the TT was prolonged, the Clauss fibrinogen decreased, and there was a mild multifactorial deficit. Treatment with Vitamin K, CCP and fibrinogen was started with temporary improvement in factor deficits. It was decided to perform BMO: 5% of pathological PCs. Congo red staining was negative. Cardiac MRI ruled out AL. The patient started a new line of treatment and a few days later, the haemorrhagic symptoms improved and the patient was discharged. Two months later, she was readmitted due to suspected sepsis abdominal. Abdominal CT shows nonspecific recto sigmoiditis and partial occlusion of the suprahepatic veins. The patient died a few days after admission. Summary/Conclusion: Conclusions: Although classic manifestations of MM (CRAB) are the most common at diagnosis of the disease, it is important to keep in mind the haemostatic alterations and haemorrhagic diathesis in these patients. Although we do not know the exact pathophysiological mechanisms, it seems that the monoclonal protein interferes at different points of coagulation, which can lead to a fibrin clot with an abnormal structure, factor deficiencies, hypo / dysfibrinogenemia or hyperfibrinolysis. Acquired factor X deficiency is widely described in the literature associated with amyloidosis (AL). However, its presence in MM patients without AL is extremely rare and there are few cases described in the literature. We must suspect factor X deficiency in the presence of a TP and APTT prolongation that is corrected with mixing tests. Treatment of acquired factor X deficiency is the treatment of the cause that originates it. In the prevention and treatment of acute bleeding, administration of exogenous factor X is indicated. If not available, CCP or PFC can be used.